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Dexamethasone- cyclophosphamide pulse in collagen vascular diseases: An observation
Sudip Das1, Parag Prasun Giri, Aloke Kr Roy
1Department of Dermatology and Venerology, NRS Medical College, Kolkata, India.
Insights
Dexamethasone-cyclophosphamide pulse (DCP) therapy shows significant improvement in various autoimmune diseases like systemic sclerosis, SLE, and dermatomyositis. Early intervention with DCP is associated with better patient outcomes and is a cost-effective treatment option.
Area of Science:
- Rheumatology
- Immunology
- Dermatology
Background:
- Collagen vascular diseases, including systemic sclerosis, dermatomyositis, and systemic lupus erythematosus (SLE), present significant treatment challenges.
- The success of dexamethasone-cyclophosphamide pulse (DCP) in pemphigus has led to its exploration in other autoimmune conditions.
Purpose of the Study:
- To evaluate the efficacy and safety of dexamethasone-cyclophosphamide pulse (DCP) therapy in patients with systemic sclerosis, dermatomyositis, SLE, and overlap syndromes.
- To compare DCP with other treatment modalities, such as methylprednisolone pulse and daily steroid regimens.
Main Methods:
- DCP was administered for six to nine pulses according to a standard regimen.
- Immunosuppressive therapy duration varied: 12-18 months for dermatomyositis, SLE, and overlap syndromes; 12 months for systemic sclerosis.
- Oral steroid doses were gradually tapered off throughout the treatment period.
Main Results:
- Significant improvements were observed in systemic sclerosis (skin binding 90%, exertional dyspnea 80%, dysphagia 40%), with minimal effect on Raynaud's phenomenon and digital tip ulcerations.
- In SLE patients, malar rash (70%), joint pain (80%), oral ulcerations (80%), fever (98%), and photosensitivity (33%) showed improvement.
- Dermatomyositis patients experienced improvements in muscle tenderness (100%), proximal myopathy (80%), heliotrope rash (80%), and shawl sign (80%).
- Overall, 25-30% of patients experienced side effects including hypertension, hyperglycemia, and infections.
Conclusions:
- Dexamethasone-cyclophosphamide pulse (DCP) is a relatively safe, effective, and inexpensive alternative to methylprednisolone pulse therapy.
- DCP is associated with fewer side effects compared to daily steroid regimens.
- Early initiation of DCP therapy correlates with improved patient response.
Background:
Treatment of collagen vascular diseases like systemic sclerosis, dermatomyositis, systemic lupus erythematosus (SLE) and even overlap syndromes has been difficult since long. Monumental success of dexamethasone-cyclophosphamide pulse (DCP) in pemphigus has prompted many a dermatologist to try it in other autoimmune diseases.
Materials And Methods:
DCP was given as per standard regimen for six to nine pulses. Immunosuppressives were given for 12-18 months in dermatomyositis, SLE, and overlap syndrome, and for 12 months in systemic sclerosis. Daily dose of steroid was tapered off gradually.
Results:
The treatment resulted in 90% improvement in skin binding in systemic sclerosis, 80% in exertional dyspnea, 40% in dysphagia, but minimum improvement was seen in Raynauds and digital tip ulcerations. No improvement in pigmentation was noted. In SLE, malar rash cleared in 70%, joint pain in 80%, oral ulcerations reduced in 80%, fever in 98%, and photosensitivity improved in one-third of patients. In dermatomyositis, improvement in muscle tenderness was seen in 100%, improvement in proximal myopathy and heliotrope rash in 80%, and improvement of shawl sign was observed in 80% of the patients. Some flattening of Gottron papules and plaques was noted in some patients. Both overlap patients improved significantly. Out of 24 patients, three were lost to follow-up, one resorted to homeopathic medicine and two expired (one dermatomyositis, one SLE). Side effects like hypertension, hyperglycemia, pyoderma, fungal infections, obesity, psychosis, etc. were seen in 25-30% of patients.
Conclusions:
We conclude that DCP is relatively safe, effective as well as cheap compared to methylprednisolone pulse. Side effects are also less compared to daily regimen of steroids. We also observed that patients who reported early and put on pulse early responded better.
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